Search
Now showing items 1-10 of 45
Loss of dystrophin is associated with increased myocardial stiffness in a model of left ventricular hypertrophy.
(Springer, 2017-03-18)
Transition from compensated to decompensated left ventricular hypertrophy (LVH) is accompanied by functional and structural changes. Here, the aim was to evaluate dystrophin expression in murine models and human subjects ...
Biomarker-driven phenotyping in Parkinson's disease: A translational missing link in disease-modifying clinical trials.
(Wiley, 2017-02-24)
Past clinical trials of putative neuroprotective therapies have targeted PD as a single pathogenic disease entity. From an Oslerian clinicopathological perspective, the wide complexity of PD converges into Lewy bodies and ...
The early onset of type 1 autoimmune hepatitis has a strong genetic influence: role of HLA and KIR genes.
(Springer Nature Switzerland AG. Springer is part of Springer Nature, 2016-02-18)
We have previously reported a strong association between HLA-DRB1*1301 and type 1 pediatric autoimmune hepatitis (PAH) and between HLA-DR*0405 and adult autoimmune hepatitis (AAH). Because human killer cell immunoglobulin-like ...
Treatment of Fabry's Disease with the Pharmacologic Chaperone Migalastat.
(Massachusetts Medical Society, 2016-08-11)
BACKGROUND:
Fabry's disease, an X-linked disorder of lysosomal α-galactosidase deficiency, leads to substrate accumulation in multiple organs. Migalastat, an oral pharmacologic chaperone, stabilizes specific mutant forms ...
Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients.
(John Wiley & Sons, Inc. All rights reserved, 2016-03-01)
BACKGROUND:
The encephalomyopathic mtDNA depletion syndrome with methylmalonic aciduria is associated with deficiency of succinate-CoA ligase, caused by mutations in SUCLA2 or SUCLG1. We report here 25 new patients with ...
International variations in application of the best-interest standard across the age spectrum.
(Springer Nature [academic journals on nature.com], 2017-02-01)
OBJECTIVE:
Ethically and legally, assertions that resuscitation is in a patient's best interest should be inversely correlated with willingness to forego intensive care (and accept comfort care) at the surrogate's request. ...
Clinical epidemiology of acute hepatitis C in South America.
(Wiley, 2016-06)
There is scarce data pertaining to acute hepatitis C (aHC) infection in South America. We aimed to describe clinical characteristics and evolution of aHC in a South American cohort. A retrospective survey was conducted at ...
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).
(Taylor & Francis Group's, 2016-01-21)
Many researchers, especially those new to the field, need to
determine which criteria are essential for demonstrating
autophagy, either for the purposes of their own research, or in
the capacity of a manuscript or grant ...
Children admitted to PICU after hematopoietic stem cell transplantation: A predictive model of mortality.
(Torres et al., 2019-08-16)
Abstract
Introduction: Hematopoietic stem cell transplantation (HSCT) in children is associated
with severe complications that need admission to critical care units. Mortality in this group
of patients remains high with ...
Fatty liver disease, an emerging etiology of hepatocellular carcinoma in Argentina
(Baishideng Publishing Group Inc, 2018-01-27)
Abstract
Aim: To investigate any changing trends in the etiologies of hepatocellular carcinoma (HCC) in Argentina during the last years.
Methods: A longitudinal cohort study was conducted by 14 regional hospitals ...